無脾症候群の乳児に対して腹腔鏡下食道裂孔ヘルニア根治術を施行した1例

Laparoscopic repair of esophageal hiatal hernia for infant with asplenia syndrome : A case report

◆要旨：症例は無脾症候群，内臓錯位，単心房単心室，共通房室弁，総肺静脈還流異常症の4か月，女児．生後1か月頃から嘔吐が増悪，上部消化管造影検査にて右側に存在する胃と食道裂孔ヘルニア，胃食道逆流を認めた．生後4か月，体重4.3kgで腹腔鏡下食道裂孔ヘルニア根治術を施行．対称肝であり肝十二指腸靱帯や胆囊が正中に存在し，胃が右側に存在するため，ポート配置は通常と左右対称かつ右側寄りとし，Nathanson liver retractorを右側腹部から挿入し，肝を挙上した．縦郭内に脱出した胃を還納し，食道裂孔縫縮後に噴門形成術（Toupet法）を行った．特徴的な解剖を把握し術野展開を工夫することで，無脾症候群の乳児でも腹腔鏡手術が可能である．

　This report describes the case of a 4-month-old girl with hiatal hernia complicated by heterotaxy with asplenia. She was born at 33 weeks of gestation and weighed 1821g. The diagnosis of heterotaxy with

asplenia and congenital cardiovascular malformations, including single ventricle with single atrium, common atrioventricular valve, total anomalous pulmonary venous connection was made postnatally. The symptom of vomiting appeared around the age of 1 month and she was referred to our department. The upper gastrointestinal series revealed right-sided stomach, hiatal hernia, and severe gastrointestinal reflux. Laparoscopic repair of the hiatal hernia was performed at 4 months old when she weighed over 4kg.

　We inserted the trocar in the reversed position compared to the typical case because of her unique morphology; the stomach presented in the right side, the liver was symmetric, and the hepatoduodenal ligament and gallbladder were located in the midline of the body. The Nathanson liver retractor was introduced from the right upper quadrant of the abdomen to elevate the symmetric liver. The right-sided stomach, which had prolapsed into the mediastinum, was repositioned. After suturing of the esophageal hiatus, Toupet fundoplication was performed. The postoperative course was uneventful.

　Accurate understanding of the unique anatomy of asplenia and the technique to develop the surgical field properly enables the performance of laparoscopic repair of the hiatal hernia safely in infants with asplenia.